Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects.

OBJECTIVES: To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible. METHODS: We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and 2008. Cases diagnosed with double-inlet LV, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia and those with hypoplastic left heart syndrome (HLHS) were included in the study population. Patients with HLHS were identified prenatally as being standard risk or high risk (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia and/or LV coronary artery sinusoids). Patients with an address over 200 miles from the hospital, diagnosed with heterotaxy syndrome or referred for fetal intervention, were excluded. RESULTS: We identified 312 cases of single-ventricle cardiac defect (208 dominant RV; 104 dominant LV) that were diagnosed prenatally. Most (96%) patients with a dominant RV had HLHS. Among the total 312 cases there were 98 (31%) elective terminations of pregnancy (TOP), 12 (4%) cases of spontaneous fetal demise, 12 (4%) cases lost to prenatal follow-up and 190 (61%) live births. Among the 199 patients that underwent fetal echocardiography before 24 weeks' gestation, there were 97 (49%) cases of elective TOP. There was no difference in prenatal outcome between those with a dominant RV and those with a dominant LV (P = 0.98). Of the 190 live births, five received comfort care. With an average of 7 years' follow-up (to obtain data on the Fontan procedure), transplantation-free survival was lower in those with a dominant RV than in those with a dominant LV (standard-risk HLHS odds ratio (OR), 3.0 (P = 0.01); high-risk HLHS OR, 8.8 (P < 0.001)). CONCLUSIONS: The prenatal outcome of cases with single-ventricle cardiac defects was similar between those with a dominant RV and those with a dominant LV, however postnatal intermediate-term survival favored those with a dominant LV. High-risk HLHS identified prenatally was associated with the lowest transplantation-free survival.

A risk-prediction model for in-hospital mortality after heart transplantation in US children.

We sought to develop and validate a quantitative risk-prediction model for predicting the risk of posttransplant in-hospital mortality in pediatric heart transplantation (HT). Children <18 years of age who underwent primary HT in the United States during 1999-2008 (n = 2707) were identified using Organ Procurement and Transplant Network data. A risk-prediction model was developed using two-thirds of the cohort (random sample), internally validated in the remaining one-third, and independently validated in a cohort of 338 children transplanted during 2009-2010. The best predictive model had four categorical variables: hemodynamic support (ECMO, ventilator support, VAD support vs. medical therapy), cardiac diagnosis (repaired congenital heart disease [CHD], unrepaired CHD vs. cardiomyopathy), renal dysfunction (severe, mild-moderate vs. normal) and total bilirubin (≥ 2.0, 0.6 to <2.0 vs. <0.6 mg/dL). The C-statistic (0.78) and the Hosmer-Lemeshow goodness-of-fit (p = 0.89) in the model-development cohort were replicated in the internal validation and independent validation cohorts (C-statistic 0.75, 0.81 and the Hosmer-Lemeshow goodness-of-fit p = 0.49, 0.53, respectively) suggesting acceptable prediction for posttransplant in-hospital mortality. We conclude that this risk-prediction model using four factors at the time of transplant has good prediction characteristics for posttransplant in-hospital mortality in children and may be useful to guide decision-making around patient listing for transplant and timing of mechanical support.

Improved survival in pediatric heart transplant recipients: have white, black and Hispanic children benefited equally?

We assessed whether the improvement in posttransplant survival in pediatric heart transplant (HT) recipients during the last two decades has benefited the major racial groups in the United States equally. We analyzed all children <18 years of age who underwent their first HT in the US during 1987-2008. We compared trends in graft loss (death or retransplant) in white, black and Hispanic children in five successive cohorts (1987-1992, 1993-1996, 1997-2000, 2001-2004, 2005-2008). The primary endpoint was early graft loss within 6 months posttransplant. Longer-term survival was assessed in recipients who survived the first 6 months. The improvement in early posttransplant survival was similar (hazard ratio [HR] for successive eras 0.80, 95% confidence interval [CI] 0.7, 0.9, p = 0.24 for black-era interaction, p = 0.22 for Hispanic-era interaction) in adjusted analysis. Longer-term survival was worse in black children (HR 2.2, CI 1.9, 2.5) and did not improve in any group with time (HR 1.0 for successive eras, CI 0.9, 1.1, p = 0.57; p = 0.19 for black-era interaction, p = 0.21 for Hispanic-era interaction). Thus, the improvement in early post-HT survival during the last two decades has benefited white, black and Hispanic children equally. Disparities in longer-term survival have not narrowed with time; the survival remains worse in black recipients.

Trends in wait-list mortality in children listed for heart transplantation in the United States: era effect across racial/ethnic groups.

We sought to evaluate trends in overall and race-specific pediatric heart transplant (HT) wait-list mortality in the United States (US) during the last 20 years. We identified all children <18 years old listed for primary HT in the US during 1989-2009 (N = 8096, 62% White, 19% Black, 13% Hispanic and 6% Other) using the Organ Procurement and Transplant Network database. Wait-list mortality was assessed in four successive eras (1989-1994, 1995-1999, 2000-2004 and 2005-2009). Overall wait-list mortality declined in successive eras (26%, 23%, 18% and 13%, respectively). The decline across eras remained significant in adjusted analysis (hazard ratio [HR] 0.70 in successive eras, 95% confidence interval [CI], 0.67-0.74) and was 67% lower for children listed during 2005-2009 versus those listed during 1989-1994 (HR 0.33; CI, 0.28-0.39). In models stratified by race, wait-list mortality decreased in all racial groups in successive eras. In models stratified by era, minority children were not at higher risk of wait-list mortality in the most recent era. We conclude that the risk of wait-list mortality among US children listed for HT has decreased by two-thirds during the last 20 years. Racial gaps in wait-list mortality present variably in the past are not present in the current era.

Racial and ethnic differences in mortality in children awaiting heart transplant in the United States.

Racial differences in outcomes are well known in children after heart transplant (HT) but not in children awaiting HT. We assessed racial and ethnic differences in wait-list mortality in children <18 years old listed for primary HT in the United States during 1999-2006 using multivariable Cox models. Of 3299 listed children, 58% were listed as white, 20% as black, 16% as Hispanic, 3% as Asian and 3% were defined as 'Other'. Mortality on the wait-list was 14%, 19%, 21%, 17% and 27% for white, black, Hispanic, Asian and Other children, respectively. Black (hazard ratio [HR] 1.6, 95% confidence interval [CI] 1.3, 1.9), Hispanic (HR 1.5, CI 1.2, 1.9), Asian (HR, 2.0, CI 1.3, 3.3) and Other children (HR 2.3, CI 1.5, 3.4) were all at higher risk of wait-list death compared to white children after controlling for age, listing status, cardiac diagnosis, hemodyamic support, renal function and blood group. After adjusting additionally for medical insurance and area household income, the risk remained higher for all minorities. We conclude that minority children listed for HT have significantly higher wait-list mortality compared to white children. Socioeconomic variables appear to explain a small fraction of this increased risk.

Balloon dilation angioplasty of peripheral pulmonary stenosis associated with Williams syndrome.

BACKGROUND: Experience of balloon dilation of peripheral pulmonary stenosis (PPS) in Williams syndrome (WS) is limited. METHODS AND RESULTS: Catheterizations in all patients with WS undergoing therapy for PPS from 1984 to 1999 were reviewed. Criteria for successful dilation included an increase >50% in predilation diameter and a decrease >20% in ratio of right ventricular (RV) to aortic (Ao) systolic pressure. Median age and weight were 1.5 years and 9.5 kg. There were 134 dilations during 39 procedures in 25 patients. The success rate for initial dilations was 51%. In multivariate analysis, successful dilation was more likely (1) in distal than in central pulmonary arteries (P=0.02), (2) if the balloon waist resolved with inflation (P=0.001), and (3) with larger balloon/stenosis ratio (P<0.001). RV pressure was unchanged after dilation (96+/-30 versus 97+/-31 mm Hg), primarily because of failure to enlarge central pulmonary arteries. The Ao pressure increased (102+/-14 versus 109+/-19 mm Hg, P=0.03), and the RV/Ao pressure ratio decreased (0.97+/-0.34 versus 0.91+/-0.30, P=0.05). Aneurysms developed after 24 dilations (18%) and were not related to balloon/stenosis ratio. Balloon rupture in 12 dilations produced an aneurysm in all 7 cases when rupture was in a hypoplastic segment. Three patients died, none from pulmonary artery trauma, and all before 1994. CONCLUSIONS: Mortality occurred early in our experience. Despite successful dilation of distal pulmonary arteries, there was modest initial hemodynamic improvement, mainly because of persistent central pulmonary artery obstruction. A serial approach of distal dilations followed by surgical repair of proximal obstruction may be a rational and successful therapy.

Predictors of outcome of biventricular repair in infants with multiple left heart obstructive lesions.

BACKGROUND: Decisions regarding surgical strategy in patients with multiple left heart obstructive or hypoplastic lesions often must be made in the newborn period and are seldom reversible. Predictors of outcome of biventricular repair have not been well defined in this heterogeneous group of patients, and risk factors described for critical aortic valve stenosis have been shown to be inapplicable to patients with other left heart obstructive lesions. The goal of this study was to identify echocardiographic predictors of outcome of biventricular repair for infants with multiple left heart obstructive lesions. METHODS AND RESULTS: Patients with >/=2 areas of left heart obstruction or hypoplasia, diagnosed at

Clinical outcome of fenestrated Fontan patients after closure: the first 10 years.

BACKGROUND: The late clinical status of Fontan patients after fenestration closure is unknown. Data are now available on all patients who underwent closure from 1989 to 1999. METHODS AND RESULTS: All patients who underwent catheter closure of a Fontan fenestration were enrolled in either the Clamshell (1989 to 1994) or CardioSEAL (1996 to 1999) regulatory trials. Physiological values obtained at catheterization helped assess the hemodynamic effects of fenestration occlusion. In addition to survival, outcomes assessed included O(2) saturations, medication use, significant clinical findings (eg, heart failure, protein-losing enteropathy, or new arrhythmias), and somatic growth. Of 181 patients who underwent closure, 27 had additional significant leaks. The remaining 154 patients constituted the study group. Median time from closure to latest follow-up was 3.4 years (range 0.4 to 10.3 years). Fenestration closure increased O(2) saturation 9.4% on average (P:<0. 001). The numbers of patients receiving digoxin or diuretics decreased at the most recent follow-up compared with baseline (P:<0. 001), but use of antiarrhythmic agents increased marginally (P:=0. 05). Height and weight percentiles rose (medians of 2 and 4, respectively; P:<0.001). Clinical decompensation during follow-up of 154 patients was rare (4.5%), with 2 deaths, 3 Fontan revisions, and 1 patient each with protein-losing enteropathy and ascites. No other patient developed chronic congestive symptoms; 21 patients developed new arrhythmias, and 2 had a stroke or transient ischemic attack. CONCLUSIONS: Fenestration closure in Fontan patients was followed by improved oxygenation, reduced need for anticongestive medication, and improved somatic growth at latest follow-up. Death (1.3%) or chronic decompensation (3.2%) was rare.

Cognitive development after the Fontan operation.

BACKGROUND: Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure. METHODS AND RESULTS: A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3. 7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7+/-17.4 (P<0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores <70 (P=0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (P=0.002), the anatomic diagnoses of hypoplastic left heart syndrome (P<0.001) and "other complex" (P=0.05), and prior placement of a pulmonary artery band (P=0.04). Mean composite achievement score was 91.6+/-15. 4 (P<0.001 versus normal); 14 patients (10.8%) scored <70 (P<0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (P=0.004) and "other complex" (P=0.003) or prior use of circulatory arrest (P=0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (P=0.01). CONCLUSIONS: Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population.

Evolution of risk factors influencing early mortality of the arterial switch operation.

OBJECTIVES: The present study was undertaken to determine the independent risk factors for early mortality in the current era after arterial switch operation (ASO). BACKGROUND: Prior reports on factors affecting outcome of the ASO demonstrated that abnormal coronary arterial patterns were associated with increased risk of early mortality. As diagnostic, surgical and perioperative management techniques continue to evolve, the risk factors for the ASO may have changed. METHODS: All patients who underwent the ASO at Children's Hospital, Boston between January 1, 1992 and December 31, 1996 were included. Hospital charts, echocardiographic and cardiac catheterization data and operative reports of all patients were reviewed. Demographics and preoperative, intraoperative and postoperative variables were recorded. RESULTS: Of the 223 patients included in the study (median age at ASO = 6 days and median weight = 3.5 kg), 26 patients had aortic arch obstruction or interruption, 12 had Taussig-Bing anomaly, 12 had multiple ventricular septal defects, 8 had right ventricular hypoplasia and 6 were premature. There were 16 early deaths (7%), with 3 deaths in the 109 patients considered "low risk" (2.7%). Coronary artery pattern was not associated with an increased risk of death. Compared with usual coronary anatomy pattern, however, inverted coronary patterns and single right coronary patterns were associated with increased incidence of delayed sternal closure (p = 0.003) and longer duration of mechanical ventilation (p = 0.008). In a multivariate logistic regression model using only preoperative variables, aortic arch repair at a separate procedure before ASO and smaller birth weight were independent predictors of early mortality. In a second model that included both pre- and intraoperative variables, circulatory arrest time and right ventricular hypoplasia were independent predictors of early death. CONCLUSIONS: The ASO can be performed in the current era without excess early mortality related to uncommon coronary artery patterns. Aortic arch repair before ASO, right ventricular hypoplasia, lower birth weight and longer intraoperative support continue to be independent risk factors for early mortality after the ASO.

Echocardiographic predictors of coronary artery pathology in pulmonary atresia with intact ventricular septum.

Coronary artery pathology is a major determinant of treatment strategy and outcome in patients with pulmonary atresia and intact ventricular septum (PA/IVS). For this reason, infants with PA/IVS routinely undergo preoperative cardiac catheterization. The goal of this study was to identify echocardiographic predictors of coronary artery pathology in infants with PA/IVS. The initial preoperative echocardiograms of 30 consecutive infants with PA/IVS (median age at diagnosis 1 day) were reviewed for indexes predicting the degree of coronary pathology. The tricuspid valve (TV) annulus diameter Z- score was determined and evidence of abnormal flow in the coronary arteries by Doppler was evaluated. Coronary pathology was defined by angiography and graded as: 0 = no fistulae; 1 = fistulae/no right ventricular (RV)-dependent coronary arteries; 2 = fistulae with 1 RV-dependent coronary; 3 = fistulae with >/=2- vessel RV-dependent coronary arteries. Outcome was classified as: 2 ventricles, "1.5" ventricles, and 1 ventricle. By angiography, 30% of the patients had grade 0 coronary pathology, 30% had grade 1, 20% had grade 2, and 20% had grade 3. There was 1 death in a patient with grade 3 coronary pathology. Among the survivors (median age at follow-up 28. 6 months), biventricular circulation existed in 12 patients (41%), 7 patients (24%) were 1.5, and 10 (34%) were 1 ventricle. All patients with TV Z-score -2.5. The sensitivity, specificity, positive, and negative predictive values of TV Z-score

Development and validation of an echocardiographic model for predicting progression of discrete subaortic stenosis in children.

The clinical course of discrete subaortic stenosis (DSS) varies considerably between patients. This study was performed to identify echocardiographic characteristics of DSS that distinguish progressive from nonprogressive disease. The study included 100 patients from 2 institutions and was performed in 2 stages. In phase I, a prediction model was developed based on multivariate analysis of morphometric and Doppler variables obtained from the initial echocardiogram in 52 children with DSS from Texas Children's Hospital. In phase II, the performance characteristics of the prediction model were tested in 48 patients with DSS followed at Children's Hospital in Boston. Patients were divided into 3 outcome groups: nonprogressive, progressive, and intermediate progression. In phase I, multivariate analysis identified 3 independent predictors of progressive disease: indexed aortic valve to subaortic membrane distance, anterior mitral leaflet involvement, and initial Doppler gradient. The logistic regression equation--Probability = [1 + e-(-322+0.334X1+4.06X2-0.708X3)](-1), where X = initial gradient in mm Hg; X2 = absence (0) or presence (1) of mitral leaflet involvement; and X3 = indexed distance between aortic valve and subaortic membrane in mm/body surface area0.5 were used to predict progression. When the prediction model was applied to phase II study patients, none of the patients with nonprogressive DSS had a prediction value > 0.29 and none of the patients with progressive DSS had a prediction value < 0.58. Thus, a prediction value > 0.55 yielded a 100% sensitivity and 100% specificity for distinguishing progressive from nonprogressive DSS. Patients with intermediate progression were indistinguishable from progressive DSS but were clearly separable from nonprogressing patients. We conclude that progressive subaortic obstruction in children with DSS can be predicted from morphologic, morphometric, and Doppler echocardiographic analysis of left ventricular outflow.

Sudden death in patients after transcatheter device implantation for congenital heart disease.

To explore whether transcatheter intracardiac devices increase risk for sudden death, we analyzed patient and device-related variables with a case-control design. Sudden death was defined as unexpected death with abrupt onset and rapid deterioration. Cases included all patients known to have died suddenly after device closure at our institution, using 3 large databases. Controls (2:1), matched on year of procedure, were chosen randomly from the same databases. Data were obtained from medical record review. Of 777 patients who received implants between February 1989 and February 1999, 9 were known to have died suddenly (1.2%). In 27 cases and controls, diverse defects were occluded: atrial (n = 16), ventricular (n = 4), Fontan fenestration (n = 4), or other (n = 3). Cases were more likely to have had a history of serious arrhythmias (p = 0.008), severe valve regurgitation (p = 0.03), > or =1 cardiac surgery (p = 0.009), and multiple devices (p = 0.03). Cases were somewhat more likely to have pulmonary hypertension (p = 0.09), ventricular dysfunction (p = 0.09), and nonatrial septal defects (p = 0.10). Patients were less likely to have device arm fractures (p = 0.05). The following were not related to sudden death: age, weight, gender, low systemic cardiac index, end-diastolic pressure, major noncardiac disease, serious adverse events, maximum device size, malposition, and residual leak. This study suggests that sudden death was more likely to occur in device-implanted patients who had severe underlying cardiac disease and/or multiple devices. No other device-related variables were associated with sudden death.

Comparison of outcome when hypoplastic left heart syndrome and transposition of the great arteries are diagnosed prenatally versus when diagnosis of these two conditions is made only postnatally.

We sought to determine the impact of prenatal diagnosis on the perioperative outcome of newborns with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). All neonates with HLHS or TGA encountered at Children's Hospital, Boston, Massachusetts, from January 1988 to May 1996 were identified and outcomes documented. Birth characteristics, preoperative, operative, and postoperative variables of term newborns with a prenatal diagnosis of HLHS or TGA who underwent a Norwood operation (n = 27) or arterial switch operation (n = 14), respectively, were compared with newborns with a postnatal diagnosis of HLHS (n = 47) or TGA (n = 28) who had undergone surgery. Of 217 neonates with HLHS and 422 with TGA, 39 and 16, respectively, had a prenatal diagnosis. The preoperative mortality among neonates aggressively managed did not differ between the prenatal and postnatal diagnosis groups for either HLHS or TGA (p >0.05). Neonates with a prenatal diagnosis who underwent surgery had objective indicators of lower severity of illness preoperatively, including a higher lowest recorded pH (p = 0.03), lower maximum blood urea nitrogen (p = 0.002), and creatinine (p = 0.03) among newborns with HLHS, and a tendency toward higher minimum of partial pressure of arterial oxygen in the TGA group (p = 0.06). Prenatal diagnosis was not associated with an improved postoperative course or operative mortality (p <0.05) within a diagnostic group. Thus, a prenatal diagnosis improves the preoperative condition of neonates with HLHS and TGA, but may not significantly improve preoperative mortality or early postoperative outcome among neonates managed at a tertiary care center.

Long-term outcome in patients with pacemakers following the Fontan operation.

Patients with pacemakers after Fontan surgery compared favorably with nonpaced patients with respect to survival. In patients with atrioventricular block, dual chamber pacing was superior to VVI pacing.

Factors that influence the development of atrial flutter after the Fontan operation.

OBJECTIVES: Atrial flutter is a frequent, potentially fatal complication of the Fontan operation, but risk factors for its development are ill defined. We evaluated clinical features that might predict the development of atrial flutter in patients who had a Fontan operation. METHODS: We evaluated 334 early survivors of a Fontan operation done between April 1973 and July 1991 (mean follow-up, 5.0 +/- 3.8 years). Evaluation included electrocardiography, Holter monitor recordings, and chart review. Modifications of the Fontan operation included an extracardiac conduit (n = 43), an atriopulmonary anastomosis (n = 117), or a total cavopulmonary anastomosis (n = 174). Patient, time, and procedure-related variables were analyzed with respect to the development of atrial flutter. RESULTS: Atrial flutter was identified in 54 (16%) patients at a mean of 5.3 +/- 4.7 years (range 0 to 19.7 years) after Fontan operation. Atrial flutter developed sooner and was more likely to occur in patients who were older at the time of Fontan operation (12.4 +/- 7.6 vs 6.3 +/- 5.2 years; p < 0.001), had a longer follow-up interval (8.7 +/- 3.9 vs 4.4 +/- 3.4 years; p < 0.001), had a prior atrial septectomy or pulmonary artery reconstruction (p < 0.01), and had worse New York Heart Association class symptoms (p < 0.02). The presence of sinus node dysfunction was associated with a higher incidence of atrial flutter (p < 0.001). Although there was a lower prevalence of atrial flutter in those patients with a total cavopulmonary anastomosis, the follow-up for this group was shorter. Anatomic diagnoses, perioperative hemodynamics, and other previous palliative operations were not associated with an increased incidence of atrial flutter. Multivariate analysis identified age at operation, duration of follow-up, extensive atrial baffling, and type of repair as factors associated with the development of atrial flutter after Fontan operation. CONCLUSION: Atrial flutter continues to develop with time after the Fontan operation. Further follow-up is necessary to determine whether a total cavopulmonary anastomosis reduces the incidence of atrial flutter.

Functional outcome after the Fontan operation: factors influencing late morbidity.

OBJECTIVES: The purpose of this study was to describe the functional outcome of a large number of patients after modifications of the Fontan operation and to investigate perioperative risk factors that might influence late functional state. METHODS: A comprehensive cross-sectional review of the first 500 patients undergoing a Fontan operation at our institution was undertaken. Those surviving with an intact Fontan circulation were reviewed by questionnaire to assess functional status and medication history. Medical records, chest roentgenograms, echocardiograms, cardiac catheterizations, and laboratory investigations were also reviewed to assess postoperative status. RESULTS: Three hundred sixty-three long-term survivors with an intact Fontan circulation were identified during cross-sectional follow-up. Median age at operation was 5.0 years (range 0.4 to 31 years), and median follow-up was 5.4 years (range 1.7 to 20 years). Most patients (91.1%) were in New York Heart Association class I or II. In a multivariate model, poor (class III or IV) functional state was associated with longer duration of follow-up (p < 0.001), a prior atrial septectomy (p = 0.03), and a prior main pulmonary artery-ascending aorta anastomosis (p = 0.05). CONCLUSIONS: A poor functional outcome is uncommon after the Fontan operation but becomes more frequent with increasing duration of follow-up.

Twenty-five-year experience with rastelli repair for transposition of the great arteries.

OBJECTIVE: Our purpose was to describe the outcome of the Rastelli repair in D -transposition of the great arteries and to determine the risk factors associated with unfavorable events. METHODS: From March 1973 to April 1998, 101 patients with D -transposition of the great arteries and ventricular septal defect underwent a Rastelli type of repair. Median age and weight were 3.1 years (10th to 90th percentiles 0.3-9.9 years) and 12.8 kg (5.9-28.2). Pulmonary stenosis was present in 73 patients and pulmonary atresia in 18; 10 patients had no left ventricular outflow tract obstruction. RESULTS: There were 7 early deaths (7%) and no operative deaths in the last 7 years of the study. Risk factors for early death, by univariable analysis, included straddling tricuspid valve (P =.04) and longer aortic crossclamping times (P =.04). At a median follow-up of 8.5 years, there were 17 late deaths and 1 patient had undergone heart transplantation. Forty-four patients had reoperations for conduit stenosis, 11 for left ventricular outflow tract obstruction, and 28 had interventional catheterization to relieve conduit stenosis. Nine patients had late arrhythmias, and there were 5 sudden deaths. Overall freedom from death or transplantation (Kaplan-Meier) was 82%, 80%, 68%, and 52% at 5, 10, 15, and 20 years, respectively. Freedom from death or reintervention (catheterization or surgical treatment) was 53%, 24%, and 21% at 5, 10, and 15 years of follow-up, respectively. CONCLUSIONS: The Rastelli repair can be performed with low early mortality. However, substantial late morbidity and mortality are associated with conduit obstruction, left ventricular outflow tract obstruction, and arrhythmia.

Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

OBJECTIVES: The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. METHODS: The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. RESULTS: The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). CONCLUSIONS: These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.

Validation of relative value scale for congenital heart operations.

BACKGROUND: To determine the validity of the newly assigned work relative value unit (RVU) scale for surgical procedures for congenital heart disease, we measured its relationship to length of hospital stay, total hospital charges, and mortality. METHODS: We identified cases by the presence of ICD-9-CM codes in nine statewide, administrative hospital discharge abstract databases for 1992. Computer algorithms were generated to assign RVUs to individual cases. Spearman correlation coefficients between work and practice expense RVUs and median length of hospital stay, total hospital charges, and in-hospital mortality were determined, as well as parameter estimates from linear and logistic regression. RESULTS: Using data from 5,192 cases involving 34 surgical procedures for congenital heart disease, higher work RVUs were associated with longer lengths of hospital stay (rs = 0.72, p < 0.0001), higher total hospital charges (rs = 0.81, p < 0.0001), and higher in-hospital mortality (rs = 0.45, p = 0.01). A 5-point increase in the relative value scale was associated with an increase in the length of stay by a multiplicative factor of 1.3 (p < 0.0001); total hospital charges by 1.5 (p < 0.0001); and the odds of in-hospital death by 1.9 (p < 0.0001). Findings were similar for practice expense RVUs, as work and practice expense RVUs were highly correlated (rs = 0.93, p < 0.0001). CONCLUSIONS: The group of work RVUs for surgical procedures for congenital heart defects are reasonable relative measures, on average, of physician work for these procedures, thus supporting the use of this scale to determine physician reimbursement. Practice expense RVUs may not be an independent measure for these procedures.